Vascular Rings

• Developmental abnormality
  • Aberrant right subclavian artery from descending aorta
    • Most common
    • Travels behind oesophagus, incomplete ring
    • Often asymptomatic, may cause posterior compression
      • “Dysphagia Lusoria”
      • Associated with non-recurrent laryngeal nerve
  • Anomalous formation of right aortic arch with left ligamentum arteriosum and resultant retro-oesophageal left subclavian artery
    • Complete ring
  • Double aortic arch
    • Fail to remodel appropriately, which results in two aortic arches both connecting the ascending and descending aortas
    • Complete ring
  • Other causes
    • Left aortic arch with a right descending aorta and right ductus arteriosus (or ligamentum)
    • Right aortic arch with a left descending aorta and left ductus arteriosus (or ligamentum)
    • Left aortic arch with an aberrant right subclavian and a right ductus arteriosus (or ligamentum)
    • Right aortic arch with mirror-image branching and a left ductus arteriosus (or ligamentum) arising from a retroesophageal dimple pointing to the left (also described as a double aortic arch with atretic left arch)
    • Right aortic arch with retroesophageal left innominate and either a left ductus arteriosus or diverticulum

Double aortic arch Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum

Pulmonary artery sling

• Left pulmonary artery comes off right instead of main branch & courses between trachea & oesophagus
• Associated with other cardiac defects & foregut abnormalities
• All require repair to prevent tracheal or left PA stenosis

Innominate artery compression syndrome

• This syndrome occurs when the innominate (or brachiocephalic) artery originates later along the course of the transverse arch, resulting in takeoff to the left of the trachea.
• Anterior tracheal compression with associated tracheomalacia results when the aberrant innominate artery passes back to the right.